What are the types of cholangiocarcinoma?

Cholangiocarcinomas arise from the epithelial cells of the bile ducts. The majority of cholangiocarcinomas (>90 percent) are adenocarcinomas, with squamous cell carcinoma being responsible for most of the remaining cases. Distant metastases are distinctly uncommon in cholangiocarcinoma.

Cholangiocarcinoma may arise in the intrahepatic (least common), perihilar, or distal
(extrahepatic) biliary tree. Bile duct tumors that involve the common hepatic duct bifurcation are referred to as Klatskin tumors regardless of whether they arise from the intrahepatic or extrahepatic portion of the biliary tree.

What is the epidemiology of cholangiocarcinoma?

Cholangiocarcinoma generally presents between 50 and 70 years of age. However, patients with primary sclerosing cholangitis (PSC) and those with choledochal cysts present nearly two decades earlier. The incidence of cholangiocarcinoma is slightly higher in men. This probably reflects the higher incidence of PSC in men.

What are the risk factors for cholangiocarcinoma?

  • PSC is the commonest known predisposing factor for cholangiocarcinoma in the UK. It is interesting to note that the risk of developing cholangiocarcinoma is not associated with the duration of PSC.
  • Chronic intraductal gall stones.
  • Bile duct adenoma and biliary papillomatosis.
  • Caroli’s disease (cystic dilatation of ducts)
  • Choledochal cysts
  • Thorotrast (a radiological agent no longer licensed for use)
  • Smoking (increased risk in association with PSC).
  • In SE Asia, where the tumour is quite common, the associated risk factors are liver flukes (Opisthorchis viverrini and Clonorchis sinensis) and chronic typhoid carriers.

What are the clinical features of cholangiocarcinoma?

i. Painless obstructive jaundice, abdominal pain and weight loss.
ii. Cholangiocarcinoma is often associated with intermittent rather than steadily progressive jaundice.

Discuss the diagnosis of cholangiocarcinoma?

  • USS- Diagnosis should be suspected when ducts are dilated.
  • CT- abdominal lymphadenopathy is common in PSC & does not necessarily indicate malignant change.
  • Tumour markers- The value of CA 19-9 in patients with suspected cholangiocarcinoma is unclear. CEA and Ca 125 are also raised in cholangiocarcinoma.
  • Tissue diagnosis of cholangiocarcinoma is difficult because it is highly desmoplastic tumour composed of a few malignant cholangiocytes within excessive fibrous tissue. Thus cytology is positive in only 1/3rd of cases.
  • Positron emission tomography — PET scan permits visualization of cholangiocarcinomas because of the high glucose uptake of bile duct epithelium. Another role for PET is in screening patients with PSC for the presence of cholangiocarcinoma.
  • Staging laparoscopy —laparoscopy will identify 25-30% of patients as unresectable who were thought to be operable before laparoscopy. Therefore laparoscopy should be standard before operation.

Discuss the management of cholangiocarcinoma?

Surgery is the only curative treatment for patients with cholangiocarcinoma.

  • Distal cholangiocarcinomas are managed by pancreatoduodenectomy as with pancreatic head cancers.
  • Intrahepatic cholangiocarcinoma is treated by resection of the involved segments or lobe of the liver.
  • Hilar tumours- en bloc resection of the extrahepatic bile ducts and gall bladder, regional lymphadenectomy, and Roux-en-Y hepaticojejunostomy with or without right or left hepatectomy

Criteria for resectability — the traditional guidelines for resectability in US include:

  • Absence of nodal metastases or distant liver metastases.
  • Absence of invasion of the portal vein or main hepatic artery
  • Absence of extrahepatic adjacent organ invasion
  • Absence of disseminated disease

However, as a general rule, true resectability is ultimately determined at surgery, particularly with perihilar tumors.

What is the role of preoperative biliary decompression?

This is controversial. Many surgeons proceed directly to laparotomy without preoperative biliary drainage. On the other hand, there is often uncertainty as to resectability as well as the timetable of surgical evaluation and operative management in patients presenting with jaundice. As a practical issue, stents are often placed to alleviate jaundice while these issues are being settled.

What is the role of chemoradiotherapy?

Neoadjuvant or adjuvant chemotherapy or radiotherapy therapy is not shown to be beneficial in patients with cholangiocarcinoma.

What are the palliative treatment options?

  • Photodynamic therapy is emerging as an important palliative option for patients with unresectable cholangiocarcinoma.
  • Biliary stenting to relieve symptoms. Palliative radiation therapy after biliary bypass or intubation may be beneficial for some patients.
  • Patients with unresectable disease can be considered candidates for phase I and II studies of chemotherapeutic agents or biologics.

What is the prognosis?
Surgery for cholangiocarcinoma is usually extensive and have a high operative mortality (5%-10%) and low curability. There is a 9–18% five year survival for proximal bile duct lesions and 20–30% for distal lesions.
Median survival for patients with intrahepatic cholangiocarcinoma without hilar involvement is 18–30 months and 12–24 months with perihilar involvement.


  1. British Society of Gastroenterology Guidelines for the diagnosis and treatment of cholangiocarcinoma: consensus document

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